Pemphigus foliaceus and acquired haemophilia: a rare but important association with life-threatening consequences

Fatal occurrence of acquired haemophilia A in a patient with pemphigus vulgaris

“…4 No systemic symptoms suggestive of a bleeding disorder occurred until the patient experienced the acute event leading to death. We agree with Wernham et al 1 that dermatologists should be aware of AHA as a potentially lethal, albeit rare, complication of AIBD. It is our opinion that, considering the subtle clinical onset of AHA, there may be scope for monitoring aPTT during the follow-up of patients with AIBD.…”

“…The association between AIBD and AHA has been reported previously. 1,2 Notably, in our patient, differently from other cases in the literature, 1,3 AHA occurred while the underlying PV was under clinical remission, raising speculation that prednisone tapering triggered the clinical manifestation of AHA. We wish to highlight that AHA may represent a most challenging diagnosis: in our patient, the condition resembled dermatoporosis, a sign of cutaneous fragility commonly observed during topical and systemic corticosteroid therapy.…”

“…doi: 10.1111/ced.14022 Autoimmune blistering dermatoses (AIBD) and acquired haemophilia A (AHA) are autoimmune conditions that may rarely coexist. Recently, Wernham et al 1 described an interesting case of recalcitrant pemphigus foliaceus complicated by AHA, emphasizing the importance of the early detection of AHA because of its life-threatening behaviour. We report our experience of a fatal occurrence of AHA in a patient with a well-controlled pemphigus vulgaris (PV).…”